Myeloproliferative Disorders Flashcards
What cells are raised in polycythaemia vera?
Raised RBCs (sometimes only this is raised), platelets, granulocytes, mast cells
What causes hepatosplenomegaly in polycythaemia vera?
Extramedullary haematopoesis
Increased destruction of RBCs (spleen)
Polycythaemia vera signs and symptoms
Hepatosplenomegaly
Gout
Thrombotic events (e.g. hand and feet swelling)
Rosy face
Pruritis on bathing (increased mast cells release histamine in response to heat)
Risk of what rare thrombotic syndrome is raised in polycythaemia vera?
Budd-chiari syndrome (hepatic vein thrombosis)
What mutation is present in myeloproliferative disorders?
JAK2
Polycythaemia vera management?
Venesection
Hydroxyurea and interferon
JAK2 inhibitors (ruxolitinib)
Signs/symptoms of essential thrombocythaemia?
Thrombosis (stroke, numb/burning hands/feet)
Bleeding/purpurae
Splenomegaly
Why is there increased risk of bleeding in essential thrombocythaemia?
vWF is all used up
Management of essential thrombocythaemia?
Low risk - aspirin
High risk - hydroxyurea, interferon (chemo)
Signs and symptoms of primary myelofibrosis?
Bone pain
Bleeding
Anaemia
Infection
Hepatosplenomegaly (extramedullary haematopoesis)
What finding on blood film in primary myelofibrosis?
Tear drop RBCs
What happens if you try to aspirate bone marrow in primary myelofibrosis?
Might not get much fluid (dry aspirate)
Can do biopsy instead
Management of primary myelofibrosis
Stem cell transplant
Transfusion (RBCs, platelets)
Potentially chemo?
